Table 2.

Linear GAA activity responses in fibroblast mixtures (nmol · h−1 · mg−1).

Expected proportionRun 1Run 2Run 3MeanSD% of Normal
100% Pompe (GM00338)0.10.10.20.10.00.12
99% Pompe + 1% normal1.41.41.41.40.01.1
98% Pompe + 2% normal2.62.42.72.60.12.1
95% Pompe + 5% normal6.46.76.76.60.25.4
90% Pompe + 10% normal12.613.913.613.30.611.0
50% Pompe + 50% normal61.669.069.366.63.554.8
100% Normal (GM23971)122.1121.7121.1121.60.4100.0